Hyperimmunoglobulin D Syndrome

Hyperimmunoglobulin D syndrome is one of the autoinflammatory syndromes which are characterized by febrile attacks. Recently it is made possible to reach more reliable data by the possibilities that are opened up by molecular genetics to highlight the aetiopathogenesis of this group of diseases. Hyperimmunoglobulin D syndrome could present itself with a broad spectrum of symptoms. Although most of the Hyperimmunoglobulin D syndrome patients are reported from Europe and especially Dutch ancestry, case reports are presented from all over the world. Hyperimmunoglobulin D syndrome should be kept in mind for the differential diagnosis of periodic fever syndromes or before accepting an familial meditterinenan fever patient as colchichine-resistant. [Archives Medical Review Journal 2013; 22(1.000): 55-64]